Overview 

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Catalog#  BPA1003

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Lot # Check on the product label

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Size 100 μg

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Isotype IgG

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Host Rabbit

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Reactivity
Human

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Product Form Liquid

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Purification Immunogen affinity purified

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Immunogen 
A synthetic peptide (conjugated to KLH) corresponding to the region of human alpha 1-antitrypsin.

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Recommend Application
Western Blot, WB (1:500)
Other applications have not been tested.
 The optimal dilutions should be determined       by end user.

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Storage Buffer
1*PBS (pH7.4), 0.2% BSA, 40% Glycerol. Preservative: 0.05% Sodium Azide.

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Storage Instruction 
Store at 4°C after thawing (1 week). Aliquot and store at -20°C for long term (at least one year).
Avoid repeated freeze and thaw cycles.

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Background
alpha 1-antitrypsin(A1AT) is a protease inhibitor belonging to the serpin superfamily. ^[1] It is generally known as serum trypsin inhibitor. A1AT is a 52-kDa serpin with a characteristic secondary structure of beta sheets and alpha helices. Disorders of this protein include alpha 1-antitrypsin deficiency, causes the degradation especially of lung tissue, and eventually leads to characteristic manifestations of pulmonary emphysema. ^[2] As A1AT is expressed in the liver, certain mutations in the gene encoding the protein can cause misfolding and impaired secretion, which can lead to liver cirrhosis.

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Reference
1. Gettins PG (2002). "Serpin structure, mechanism, and function". Chem Rev 102 (12): 4751–804.
2. DeMeo DL, Silverman EK (2004). "α1-Antitrypsin deficiency · 2: Genetic aspects of α1-antitrypsin deficiency: phenotypes and genetic modifiers of emphysema risk". Thorax 59 (3): 259–64.